Amyloidosis with a focus on Cardiac
  1. What is Amyloidosis?
    1. The types of disease
      1. Are disease is a build up of protein (amyloid)
      2. This can occur in many different areas of the body - heart, liver, spleen, CNS, kidneys, or GI
      3. Types of Amyloidosis
        1. AL Amyloidosis (primary) - plasma cell disorder that comes from monoclonal immunoglobulin light chain. These fibrils can occur anywhere in the body
        2. AA Amyloidosis (secondary) - comes from infections and inflammation that generates the amyloid fibrils. Usually begins in the kidneys.
        3. ATTR (m or wt) Amyloidosis -
          1. ATTRm is an inherit mutation of transthyretin (TTR) protein that generates fibrils that cause cardiomyopathy or neuropathy that usually occur in people over 40
          2. There are more than 100 types of TTR
          3. ATTRwt is considered an non-mutated protein that may cause cardiomyopathy in older men
        4. There are other types of Amyloidosis which includes: Apolipoproteint A-I (AApoAI), Apolipoprotein A-II, Gelsolin (AGel), Fibriogen (A Fib), Lysozyme (ALys), Beta-2 Immunoglobulin Amyloidosis (Abeta2m), and Localized Amyloidosis (ALoc)
        5. Our focus is with ARRT Amyloidosis that affects the function of the myocardium
          1. ATTR is our focus and it is most commonly found in men in their 70s and to a lesser extent in their 60s
          2. One in four men have some ATTR their 80s, but it does not appear to have any effect
          3. Deposits of ARRT occur in the LV that cause myocardial wall thickening
          4. ~4% of US African Americans acquire ARRTm that may contribute to heart failure
    2. Diagnosing AL
      1. Echocardiography or MRI has been used to diagnose this disease
      2. The application of 99mTc-PYP appears to be a viable alternative assess and manage these patients
    3. Nuclear Medicine Procedure
      1. No patient preparation is needed
      2. Administer 10 to 20 mCi of 99mTc-PYP IV
      3. Delayed images can occur at 1 and/or 3 hours post dose
      4. LEHR collimator
      5. Planar imaging use 256 matrix
      6. SPECT imaging use 128 matrix
      7. Planar images
        1. ANT, L-LAT, and LAO
        2. 750k counts
        3. MAG @ 1.46
      8. SPECT (assume this is a dual headed camera)
        1. 180 degree acquisition
        2. No gating
        3. Number of stops ~40
        4. Twenty seconds per stop
        5. MAG 1.0
    4. Assessment of myocardial uptake
      1. Grade Zero = No myocardial uptake uptake + normal rib uptake
      2. Grade One = Myocardial uptake less than ribs
      3. Grade Two = Myocardial uptake equal to ribs
      4. Grade Three = Myocardial uptake greater than ribs
      5. Example of Grades 0 through 3

References
1. Types of Amyloidosis, Boston University, Amyloidosis Center. https://www.bu.edu/amyloid/what/types/
2. 99mTechnetium-Pyrophosphate Imaging for Transthyretin Cardiac Amyloidosis, ASNC - link

10/23